RESUMO
PURPOSE: To review a multicentric series of lateral-type posterior fossa ependymomas operated in the last ten years and to analyze the factors related to clinical evolution and tumor survival. METHODS: Descriptive, retrospective study. Active members of the Spanish Society of Pediatric Neurosurgery were invited to participate in this multicentric study. Clinical and radiological data were incorporated to an open database. The role of histologic grade, grade of resection, postoperative morbidities, and clinical follow-up was evaluated through bivariate associations (chi-square), Kaplan-Meier's curves (log-rank test), and multivariate analysis (binary logistic regression). RESULTS: Fourteen centers entered the study, and 25 cases with a minimum follow-up of 6 months were included. There were 13 boys and 12 girls with a mean age close to 3 years. Mean tumor volume at diagnosis was over 60 cc. A complete resection was achieved in 8 patients and a near-total resection in 5 cases. Fifteen tumors were diagnosed as ependymoma grade 2 and ten as ependymoma grade 3. Major morbidity occurred postoperatively in 14 patients but was resolved in twelve within 6 months. There were six cases of death and 11 cases of tumor progression along the observation period. Mean follow-up was 44.8 months. Major morbidity was significantly associated with histologic grade but not with the degree of resection. Overall and progression-free survival were significantly associated with complete surgical resection. At the last follow-up, 16 patients carried a normal life, and three displayed a mild restriction according to Lansky's scale. CONCLUSIONS: Lateral-type posterior fossa ependymomas constitute a specific pathologic and clinical tumor subtype with bad prognosis. Gross total resection is the goal of surgical treatment, for it significantly improves prognosis with no additional morbidity. Neurological deficits associated to lower cranial nerve dysfunction are common, but most are transient. Deeper genetic characterization of these tumors may identify risk factors that guide new treatments and stratification of adjuvant therapies.
Assuntos
Ependimoma , Masculino , Feminino , Humanos , Criança , Estudos Retrospectivos , Prognóstico , Terapia Combinada , Intervalo Livre de Progressão , Ependimoma/cirurgia , Ependimoma/patologiaRESUMO
The endoscopic transventricular transchoroidal approach facilitates entry into the posterior part of the third ventricle, allowing a visualization field from the foramen of Monro to the pineal region through this anatomical corridor. Combined surgery to treat the target lesion and possible endoscopic third ventriculostomy (ETV) can be performed through a single burr hole. A detailed description of this surgical technique is given, and a series of cases from our center is presented. This retrospective study included patients with lesions in the pineal region or posterior zone of the third ventricle who underwent surgery between 2004 and 2022 in our center for tumor biopsy or endoscopic cyst fenestration. In nine cases, the transchoroidal approach was performed. Demographic and clinical variables were collected: sex, age at diagnosis, clinical presentation, characteristics of the lesion, pathological diagnosis, characteristics of the procedure, complications, subsequent treatments, evolution, follow-up time, and degree of success of the endoscopic procedure. The mean and range of the quantitative variables and frequency of the qualitative variables were analyzed, together with the statistical significance (p < 0.05). Surgical planning was carried out by performing a preoperative MRI, calculating the ideal entry point and trajectory for each case. The preoperative planning of the surgical technique is described in detail. Of our sample, 55.6% were women, with a mean age of 35 years (7-78). The most common clinical presentation was intracranial hypertension (55.6%), with or without a focus. Eight patients presented hydrocephalus at diagnosis. The most frequent procedure was endoscopic biopsy with ETV (66.7%). The pathological diagnosis varied widely. Procedure-related complications included one case of self-limited bleeding of the choroidal fissure at its opening and one intraventricular hemorrhage due to tumor bleeding in the postoperative period. Non-procedure-related complications comprised two ETV failures and one case of systemic infection, while late complications included one case of disease progression and one case of radionecrosis. Four patients died, one due to poor neurological evolution after post-surgical tumor bleeding and three due to causes unrelated to the procedure. The rest of the patients had a favorable evolution and were asymptomatic or stable. The transchoroidal approach through a single burr hole is a feasible and safe option for access to the posterior part of the third ventricle. Proper planning of each case is necessary to avoid complications.
Assuntos
Neoplasias do Ventrículo Cerebral , Hidrocefalia , Neuroendoscopia , Terceiro Ventrículo , Adulto , Feminino , Humanos , Masculino , Neoplasias do Ventrículo Cerebral/cirurgia , Hidrocefalia/etiologia , Neuroendoscópios/efeitos adversos , Neuroendoscopia/métodos , Estudos Retrospectivos , Terceiro Ventrículo/cirurgia , Ventriculostomia/métodos , Criança , IdosoRESUMO
Although intraventricular neuroendoscopy is considered a minimally invasive technique with good results, there is nevertheless a risk of developing certain complications. As no agreement apparently exists concerning the classification of these complications, we aim to propose a form of classification based on the results of our series, comparing them with recent publications. We undertook a retrospective study of 170 children who underwent intraventricular neuroendoscopy between 2003 and September 2020 at our center. Data were recorded on demographic and clinical variables: age, gender, presenting symptoms, etiology, number of procedures, type of procedure, and complications. Complications were divided into two main groups, intraoperative and postoperative, and in subgroups. The intraoperative complications included one group of systemic alterations and another group of surgical problems. The postoperative complications were divided into six groups: systemic, neurologic, hormone, fluid, hemorrhagic and death. A total of 202 neuroendoscopic procedures were performed in 170 children. The mean age at first surgery was 71 months (22-122). The most common etiology of the hydrocephalus was intraventricular tumors (32.9%), followed by aqueductal stenosis (13.5%). The most usual presenting sign was intracranial hypertension. The procedure most used was third ventriculostomy (62.9%). During the procedures, we experienced 5 surgical intraoperative complications (2.47% per procedure). In the postoperative period, there were 23.7% systemic complications per procedure, 12.87% neurologic, 8.41% hormone, 10.9% fluid, 0.5% hemorrhagic, and 0.99% for postoperative death. The rate of complications associated with intraventricular neuroendoscopy was similar in our series to those already published. Comparative studies require standardization for the analysis of neuroendoscopic complications.
Assuntos
Hidrocefalia , Neuroendoscopia , Terceiro Ventrículo , Criança , Humanos , Hidrocefalia/diagnóstico , Hidrocefalia/etiologia , Hidrocefalia/cirurgia , Neuroendoscopia/efeitos adversos , Neuroendoscopia/métodos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgia , Padrões de Referência , Estudos Retrospectivos , Terceiro Ventrículo/cirurgia , Resultado do Tratamento , Ventriculostomia/efeitos adversos , Ventriculostomia/métodosRESUMO
BACKGROUND: Idiopathic intracranial hypertension (IIH) is a clinical syndrome characterised by raised intracranial pressure with no discernible aetiology. It is relatively rare in children and its demographic features may differ from those of adults. The relationship between IIH and venous sinus stenosis (VSS) is well known. As VSS plays an important role in the pathophysiology, treatments have been developed aimed at improving venous blood outflow in refractory IIH. In the last two decades, venous sinus stenting has emerged as a treatment option in cases where stenosis is documented. METHODS AND RESULTS: The scientific literature on paediatric cases of IIH and its treatment with venous sinus stenting was analysed. We present the case of a 6-year-old girl with a life-threatening presentation of IIH, who was treated with transverse sinus stenting and a lumboperitoneal shunt. We summarise the characteristic of paediatric stenting cases reported and review the literature focusing on the main aspects of venous sinus stenting. CONCLUSION: VSS stenting could be a treatment tool for the acute presentation of IIH with severe symptoms and VSS plus an elevated trans-stenotic pressure gradient. However, in some cases, additional surgical treatment may be necessary.
Assuntos
Hipertensão Intracraniana , Pseudotumor Cerebral , Adulto , Criança , Constrição Patológica/diagnóstico por imagem , Constrição Patológica/cirurgia , Cavidades Cranianas/diagnóstico por imagem , Cavidades Cranianas/cirurgia , Feminino , Humanos , Hipertensão Intracraniana/etiologia , Hipertensão Intracraniana/cirurgia , Pseudotumor Cerebral/complicações , Pseudotumor Cerebral/diagnóstico por imagem , Pseudotumor Cerebral/cirurgia , Estudos Retrospectivos , Stents , Resultado do TratamentoRESUMO
BACKGROUND: Rhinoliquorrhoea suggests a communication between the subarachnoid space and the sinonasal tract. Clinical presentation includes clear nasal discharge, headache, pneumocephalus, meningitis or brain abscess. Cerebrospinal fluid (CSF) leaks are mostly of traumatic origin (skull base fractures), iatrogenic (secondary to endoscopic endonasal surgery) or associated with tumour aetiology. Occasionally, hydrocephalus has been the cause of rhinoliquorrhoea in adults, presumably secondary to the chronically raised intracranial pressure with skull base erosion and meningocele. To our knowledge, the association of hydrocephalus and ethmoid meningoencephalocele/CSF leak has not been previously reported in a newborn child. CASE PRESENTATION: We present the case of a 9-month-old girl who was referred for rhinorrhoea. She had a history of posthaemorrhagic ventricular dilatation. Brain computed tomography (CT) and magnetic resonance imaging (MRI) showed a left ethmoidal meningoencephalocele and small ventricular size. The meningoencephalocele was surgically repaired using an intradural subfrontal approach. During the postoperative period, after the transient lumbar drain was withdrawn, she developed symptomatic hydrocephalus. Ventriculoperitoneal shunting was required. CONCLUSION: Progressive ventricular dilatation may arise from a meningoencephalocele/CSF leak in paediatric patients. Early identification and repair of the meningoencephalocele are critical to avoid development of complications.
Assuntos
Ventrículos Cerebrais/diagnóstico por imagem , Rinorreia de Líquido Cefalorraquidiano/diagnóstico , Encefalocele/diagnóstico , Osso Etmoide/diagnóstico por imagem , Hidrocefalia/diagnóstico , Hemorragias Intracranianas/complicações , Meningocele/diagnóstico , Ventrículos Cerebrais/patologia , Rinorreia de Líquido Cefalorraquidiano/etiologia , Ecoencefalografia , Encefalocele/etiologia , Osso Etmoide/patologia , Feminino , Humanos , Hidrocefalia/etiologia , Lactente , Imageamento por Ressonância Magnética , Meningocele/etiologia , Tomografia Computadorizada por Raios XRESUMO
The symptoms related to the presence of arachnoid cysts in the Central Nervous System depend on the size of the cyst and its growth rate, its location and, in some cases, the associated CSF dynamic disorder. Sometimes there is acute clinical presentation due to cyst rupture or acute bleeding. Although it is generally accepted that asymptomatic or paucisymptomatic cysts do not require surgical treatment, there is no consensus on the therapeutic approach of choice in symptomatic cases. The aim of this paper is to review the literature, analyzing the pros and cons of the three main surgical options (microsurgery, neuroendoscopy, and CSF shunt) based primarily on the location of the cyst. Although treatment must be always individualized, basic management recommendations may be offered.
Assuntos
Algoritmos , Cistos Aracnóideos/terapia , Cistos Aracnóideos/diagnóstico , Humanos , Guias de Prática Clínica como AssuntoRESUMO
OBJETIVES: Indication for endoscopic third ventriculostomy (ETV) in the treatment for noncommunicating hydrocephalus is widely accepted. There is controversy regarding the indication of a second procedure (re-ETV) when the first has failed. The objective of this work is to revise ETV failures in a series in which re-ETV was performed and to describe the factors related to its prognosis. METHOD: Retrospective study of pediatric patients with ETV failure treated by re-ETV between 2003 and 2018. Gender, age in first and second ETV, time to failure of first ETV, etiology of hydrocephalus, previous presence of shunt, ETV-SS in the first and second ETV, intraoperative findings, success of the second procedure and follow-up were collected. The ETV-SS result was grouped into high (≥ 80), moderate (50-70) or low (≤ 40) scores. Endoscopic procedure failure was considered clinical worsening or the absence of radiological criteria for improvement (reduction in ventricular size or presence of ETV flow artifact in the floor of third ventricle). RESULTS: Of 97 ETV carried out in this period, 47 failures were registered, with 13 re-ETV performed. Of these, 8 were classified as successful (61.53%). Re-ETV was successful in 4/4 cases in which etiology was tectal tumor or aqueduct stenosis. In the group with a high ETV-SS score there was a higher rate of success (75%) than in the group with a moderate score (40%). 9 patients presented shunt prior to first ETV and in them, success was 66.6% compared to 50% in the group without prior shunt. All re-ETV were performed without complications. In 11 of the 13 procedures a closed stoma was found and the remaining 2 cases, we found a punctate opening. The mean follow-up after re-ETV was 61.23 months. CONCLUSION: The selection of patients for re-VET should be cautious. Factors such as age, etiology, and previous shunt (ETV-SS factors) have prognostic influence. However, there are specific factors which indicate favorable prognostic for re-VET such as a longer time to failure of the first procedure, the finding of a closed/punctate stoma or the loss of flow artifact in the follow-up MRI.
Assuntos
Hidrocefalia , Neuroendoscopia , Terceiro Ventrículo , Criança , Humanos , Hidrocefalia/diagnóstico por imagem , Hidrocefalia/etiologia , Hidrocefalia/cirurgia , Neuroendoscopia/efeitos adversos , Neuroendoscopia/métodos , Reoperação/efeitos adversos , Estudos Retrospectivos , Terceiro Ventrículo/diagnóstico por imagem , Terceiro Ventrículo/cirurgia , Resultado do Tratamento , Ventriculostomia/efeitos adversos , Ventriculostomia/métodosRESUMO
OBJECTIVE: The objectives of this study were to determine the quality of life of a pediatric cohort with hydrocephalus treated by endoscopic third ventriculostomy (ETV), using the Hydrocephalus Outcome Questionnaire-Spanish version (HOQ-Sv), and study the clinical and radiological factors associated with a better or worse functional status. METHODS: This cross-sectional study was undertaken between September 2018 and December 2019. It comprised a series of 40 patients ranging from 5 to 18 years old with hydrocephalus treated by ETV. ETV was considered to be successful if there was no need for surgery for the treatment of hydrocephalus after a minimum follow-up of 6 months. The clinical variables included gender, age at hydrocephalus diagnosis, age at the time of ETV, age at completion of the questionnaire, etiology and type of hydrocephalus (communicating or not), prior shunt, repeat ETV, number of neurosurgical procedures, number of epileptic seizures, presenting signs, and follow-up duration until last office revision. The radiological variables were the Evans Index and the pre- and posttreatment frontooccipital horn ratio. An analysis was conducted of the association between all these variables and the various dimensions on the HOQ-Sv, completed by the parents of the patients via telephone or in the outpatient offices. RESULTS: The mean age of the children at ETV was 7 years (range 7-194 months), and on completing the questionnaire was 12 years (range 60-216 months). The mean HOQ scores were as follows: overall 0.82, physical domain 0.86, social-emotional (SE) domain 0.84, cognitive domain 0.75, and utility score 0.90. A history of epileptic crises was a predictive factor for a worse score overall and in the SE and cognitive domains. Factors related to a worse score in the physical domain were a previous shunt, the number of procedures, and the etiology and type of hydrocephalus. The mean follow-up duration from ETV to the last office visit was 5 years (64.5 months). No association was found between the degree of ventricular reduction and the quality of life. CONCLUSIONS: The factors related to a worse score in the different dimensions of the HOQ were a history of epileptic seizures, the number of procedures, communicating hydrocephalus, and having had a previous valve. No association was found between the reduction in ventricular size and the quality of life as measured on the HOQ-Sv.
Assuntos
Hidrocefalia/cirurgia , Neuroendoscopia/métodos , Qualidade de Vida , Terceiro Ventrículo/cirurgia , Ventriculostomia/métodos , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Estudos Transversais , Feminino , Humanos , Masculino , Resultado do TratamentoRESUMO
OBJETIVES: Indication for endoscopic third ventriculostomy (ETV) in the treatment for noncommunicating hydrocephalus is widely accepted. There is controversy regarding the indication of a second procedure (re-ETV) when the first has failed. The objective of this work is to revise ETV failures in a series in which re-ETV was performed and to describe the factors related to its prognosis. METHOD: Retrospective study of pediatric patients with ETV failure treated by re-ETV between 2003 and 2018. Gender, age in first and second ETV, time to failure of first ETV, etiology of hydrocephalus, previous presence of shunt, ETV-SS in the first and second ETV, intraoperative findings, success of the second procedure and follow-up were collected. The ETV-SS result was grouped into high (≥80), moderate (50-70) or low (≤40) scores. Endoscopic procedure failure was considered clinical worsening or the absence of radiological criteria for improvement (reduction in ventricular size or presence of ETV flow artifact in the floor of third ventricle). RESULTS: Of 97 ETV carried out in this period, 47 failures were registered, with 13 re-ETV performed. Of these, 8 were classified as successful (61.53%). Re-ETV was successful in 4/4 cases in which etiology was tectal tumor or aqueduct stenosis. In the group with a high ETV-SS score there was a higher rate of success (75%) than in the group with a moderate score (40%). 9 patients presented shunt prior to first ETV and in them, success was 66.6% compared to 50% in the group without prior shunt. All re-ETV were performed without complications. In 11 of the 13 procedures a closed stoma was found and the remaining 2 cases, we found a punctate opening. The mean follow-up after re-ETV was 61.23 months. CONCLUSION: The selection of patients for re-VET should be cautious. Factors such as age, etiology, and previous shunt (ETV-SS factors) have prognostic influence. However, there are specific factors which indicate favorable prognostic for re-VET such as a longer time to failure of the first procedure, the finding of a closed/punctate stoma or the loss of flow artifact in the follow-up MRI.
RESUMO
INTRODUCTION: Neuroendoscopy has become an effective and safe treatment for arachnoid cysts in the paediatric population. We review the paediatric patients with arachnoid cysts treated by neuroendoscopy in our hospital and analyse the results. MATERIAL AND METHODS: A retrospective analysis of 20 patients operated on from 2005 to 2018. The variables assessed are: gender, age, clinical presentation, cyst site, presence of hydrocephalus and/or extra-axial collections, endoscopic procedures and complications. Procedure success is defined as an improvement in symptoms and reduction in cyst size until end of follow-up. RESULTS: Our series comprised 13 males and 7 females (mean age: 64.6 months, range: 4-172 months). The most frequent site was suprasellar-prepontine (7), followed by intraventricular (6), quadrigeminal (3), interhemispheric (2) and Sylvian (2). A total of 70% (14/20) of patients had hydrocephalus at diagnosis, which increased to 85% in suprasellar-prepontine cysts and 100% in quadrigeminal cysts. Only 4/14 patients with required a ventriculoperitoneal shunt (median age at diagnosis: 12.5 months). Of these 4 patients, 3 developed severe shunt overdrainage. The procedure was successful in 60% (12/20) of the patients in the series. Success by location was 57% (4/7) in suprasellar cysts, 33% (1/3) in quadrigeminal cysts, 66% (4/6) in intraventricular cysts, 100% (2/2) in interhemispheric cysts and 50% (1/2) in Sylvian cysts. Treatment thus failed in 8 cases, with a mean time to failure of 12.12 months (range: 0-45 months). A new neuroendoscopic procedure was performed in 4 of these 8 cases (success in 2/4), a ventriculoperitoneal shunt was placed in 2 cases, a cystoperitoneal shunt was placed in 1 case and the remaining case was managed conservatively. Mean follow-up time was 52.45 months (range: 3-129 months). CONCLUSIONS: Neuroendoscopy is an effective and safe treatment for arachnoid cysts in paediatric patients that also enables managing associated hydrocephalus in most cases. The choice of neuroendoscopic procedure and success rate depend on cyst location. Younger patients have been found to have a higher shunt dependency rate. In these cases, measures to prevent shunt overdrainage are recommended.
Assuntos
Cistos Aracnóideos , Hidrocefalia , Neuroendoscopia , Cistos Aracnóideos/diagnóstico por imagem , Cistos Aracnóideos/cirurgia , Criança , Feminino , Humanos , Hidrocefalia/etiologia , Hidrocefalia/cirurgia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , VentriculostomiaRESUMO
OBJECTIVE: The aims of our study were to evaluate tumour response in a series of patients with vestibular schwannoma (VS) treated with linear accelerator stereotactic radiosurgery (LINAC-RS), to describe the complications and to analyze the variables associated with the response to treatment. MATERIAL AND METHODS: This retrospective descriptive study included 64 patients treated from 2010 to 2016 with a minimum follow-up of one year, excluding patients with neurofibromatosis. Clinical-radiological parameters were evaluated. The treatment was performed using LINAC-RS. The prescribed dose was 12Gy at 90% isodose. RESULTS: The mean age at treatment was 53 years, 56% were women. Ninety-eight percent of the patients had hearing loss, 71% with grade III according to the Gardner-Robertson Classification. The mean volume at treatment was 2.92cc and the mean follow-up, 40.95 months. The overall therapeutic success was 90%, reaching 100% at 12 and 24 months, and 86% after 36 months of follow-up. The radiological result was significantly related to the initial tumour volume (p<0.037). In 20 patients there was evidence of transient tumour growth compatible with pseudoprogression. Acute complications were present in 37.5%, and transitory complications in 50%. Chronic complications were found in 20%, with 84% being permanent. The rate of acute complications was lower in patients with regression (p<0.016). Chronic complications were more frequent in the 41-60 year old age group (p<0.040). CONCLUSIONS: In our study, the overall tumour control was in accordance with other published series. The radiological result significantly related to the tumour volume at the commencement of treatment. The rate of acute complications was lower in patients with regression.
Assuntos
Neuroma Acústico/radioterapia , Radiocirurgia/métodos , Adulto , Progressão da Doença , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Neuroma Acústico/diagnóstico por imagem , Neuroma Acústico/patologia , Radiocirurgia/efeitos adversos , Radiocirurgia/instrumentação , Dosagem Radioterapêutica , Estudos Retrospectivos , Resultado do Tratamento , Carga TumoralRESUMO
BACKGROUND: Atypical meningioma is a heterogeneous group of tumors with an unpredictable behavior. Our objectives were to study patients with atypical meningioma, monitor their follow-up, identify the histologic characteristics, and analyze factors associated with severe outcomes. METHODS: This retrospective study involved 28 patients with atypical meningioma from 1994 to 2014. The histologic samples were reviewed under current criteria. The association with recurrence and survival was analyzed statistically for clinical, therapeutic, histologic, and molecular factors. RESULTS: The average age at the time of diagnosis was 55.5 years (range, 18-83 years) and 57% were female. Total resection (Simpson grade I) was performed in 10 cases (38%). Adjuvant radiotherapy was given to 13 patients. During the 77-month follow-up, 64% had a recurrence or progression. The average time to first recurrence and overall survival were 43.83 months and 149 months, respectively. Recurrence was found in 85% of the patients whose samples presented necrosis. Of the tumors, 84% showed a high mitotic index (4-20 mitoses/10 high-power field). Of the 4 patients with a low mitotic index (<4 mitoses/10 high-power field), the degree of resection was subtotal and 3 experienced disease progression. Of patients with Ki67 >9.9%, 67% had recurrence. The degree of resection was the only variable significantly associated with tumor recurrence. CONCLUSIONS: The high rate of recurrence observed and the short disease-free survival exemplify the unpredictable behavior of atypical meningiomas. Total resection was the only significant factor associated with recurrence. However, the presence of atypical features, such as necrosis or high Ki67, is frequent in patients with disease progression.